Name: HPS5 Polyclonal Antibody
SKU: WP1186
Price: 268 USD
Availability: InStock

HPS5 Polyclonal Antibody

Ucallm_WP1186-50

Price range: $268.00 through $328.00

Size

100μl

50μl

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SKU: WP1186-100

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Product details

Background:

This gene encodes a protein that may play a role in organelle biogenesis associated with melanosomes, platelet dense granules, and lysosomes. This protein interacts with Hermansky-Pudlak syndrome 6 protein and may interact with the cytoplasmic domain of integrin, alpha-3. Mutations in this gene are associated with Hermansky-Pudlak syndrome type 5. Multiple transcript variants encoding two distinct isoforms have been identified for this gene. [provided by RefSeq, Jul 2008],

Specifications

Size	50μL/100μL
Target	HPS5
Reactivity	Human, Mouse
Applications	WB, ELISA
MW(Calculated)
MW(Observed)	124kD
Host Species	Rabbit
Isotype	IgG
Conjugate/Modification	Unmodified
Recommended Dilution Ratio	WB 1:500-2000; ELISA 1:5000-20000
Formulation	Liquid in PBS containing 50% glycerol,0.5% BSA and 0.02% sodium azide.
Purification	The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Storage	-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration	1 mg/ml
Clonality	Polyclonal
Clone Number
Immunogen	Synthesized peptide derived from part region of human protein
Specificity	HPS5 Polyclonal Antibody detects endogenous levels of protein.
Gene Name	HPS5 AIBP63 KIAA1017
Protein Name	Hermansky-Pudlak syndrome 5 protein (Alpha-integrin-binding protein 63) (Ruby-eye protein 2 homolog) (Ru2)
Other Name
Species	Human
Gene ID	11234
Uniprot	Q9UPZ3,
Species.1	Mouse
Gene ID.1
Uniprot.1	P59438
Species.2
Gene ID.2
Uniprot.2
Cellular Localization	Cytoplasm, cytosol .
Tissue Expression	Widely expressed. Isoform 1:Highly expressed in lungs and testis. Isoform 2:Highly expressed in placenta, kidney, testis ovary, lung and thymus.
Signaling_pathway
Research Areas
Function	Disease:Defects in HPS5 are the cause of Hermansky-Pudlak syndrome type 5 (HPS5) [MIM:203300]. Hermansky-Pudlak syndrome (HPS) is a genetically heterogeneous, rare, autosomal recessive disorder characterized by oculocutaneous albinism, bleeding due to platelet storage pool deficiency, and lysosomal storage defects. This syndrome results from defects of diverse cytoplasmic organelles including melanosomes, platelet dense granules and lysosomes. Ceroid storage in the lungs is associated with pulmonary fibrosis, a common cause of premature death in individuals with HPS.,Function:May regulate the synthesis and function of lysosomes and of highly specialized organelles, such as melanosomes and platelet dense granules. Might be involved in the regulation of general functions of integrins.,PTM:Phosphorylated upon DNA damage, probably by ATM or ATR.,similarity:Belongs to the HPS5 family.,subunit:Directly interacts with HSP6 in a complex known as biogenesis of lysosome-related organelles complex-2 (or BLOC2). Might interact with all alpha-integrin chains that have an aromatic residue before the first lysine of the conserved KXGFFKR motif, including ITGA2, ITGA3, ITGA5 and ITGA6.,tissue specificity:Widely expressed, with highest expression in liver, spinal chord, testis and thalamus.,